Intramedullary cavernous haemangioma.

S Anand, V Puri, S Sinha, V Malhotra 
 Department of Neurology, G.B. Pant Hospital, New Delhi - 110002, India., India

Correspondence Address:
S Anand
Department of Neurology, G.B. Pant Hospital, New Delhi - 110002, India.
India

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::   Introduction

Cavernous haemangiomas are vascular hamartomas and occur throughout the neuroaxis.[1],[2],[3],[4],[5],[6],[7] They are more common in cerebral hemispheres and brainstem. Spinal cavernous malformations occur most commonly in vertebrae and may extend into the extradural spinal canal. Intradural and extradural cavernomas have also been reported.[8] Intramedullary haemangiomas are extremely rare. Treatment of cavernous angioma is surgical but there are only a few reports of complete surgical excision.[9],[10] A case of intramedullary cavernous haemangioma who had complete surgical removal, is reported.

::   Case reports

Case 1 : A 35 years old male presented with nuchal pain along with numbness of both upper limbs of 4 years duration. Initially, neck pain was localised, tolerable and got precipitated on neck flexion. After two and a half years of its onset, on three occasions the neck pain became intolerable and started radiating to both upper limbs, more so on the right side. This was associated with weakness in left upper limb. He denied any gait disturbance or bladder or bowel involvement. On all the three occasions, the weakness recovered on its own within two to five weeks. However, the weakness of left arms was persistent but gradually progressive for the last 18 months. He denied any sensory impairment or sphincter involvement.

On neurological examination, there was wasting of left thenar and hypothenar muscles with fasiculations along with hypertonia in left upper and both lower limbs. The power in left upper and both lower limbs was grade 4/5 on MRC scale. His left hand grip was weak. The deep tendon reflexes were brisk. Left plantar was extensor while right was equivocal. Rest of the neurological examination as well the systemic examination was non-contributory. His routine haemogram and biochemical parameters were normal. Roentgenogram of chest and cervical spine for crainovertebral anomalies were normal. MRI of cervical spine revealed expansion of the cord from cervicomedullary region to D4. There was an intramedullary lesion at C2-C3, which was heterogeneously low to isointense with central hyper intensity on T1WI and hyper intense on T2WI with central and peripheral hypo intensity [Figure. 1],[Figure. 2]. The entire expanded portion was heterogeneously hypo intense on T1WI and hyper intense on T2WI. Electrophysiological evaluation with motor and sensory nerve conduction studies including F wave was normal. Fibrillations were detected in deltoid and forearm muscles of either side. The patient underwent laminectomy with an impression of angioma from C1 to C4. Under the operating microscope, a bluish black discolouration was seen on left side of spinal surface at the level of C2-C3. A myelotomy was done in left para median side of the bluish black looking discolouration of the spinal cord. Total excision of the mass was performed, dissecting in haemosiderin stained gliotic plane. The mass was dark blue mulberry shaped consisting of multiple cysts with old clotted blood. Histopathological examination of the

mass revealed the presence of large blood spaces lined by endothelial cells, characteristics of cavernous haemangioma. There was no interspersed neural tissue. There was no evidence of thrombosis. The patient showed improvement in the power as well in the handgrip. He did not experience any episode of pain on his follow up of one and a half year.

::   Discussion

Intramedullary cavernous malformations of spinal cord occur more frequently in females with approximate ratio of 2 : 1. In general, the presentation is in the 3rd to 6th decade of life with progressive paraparesis, sensory loss, frequently associated pain. It may be indistinguishable from chronic progressive radiculomyelopathy. This varied manifestation is attributed to its variable size, which may vary from few millimeters to several centimeters. Acute symptoms are probably caused by new haemorrhages within or around the lesion. Slowly, progressive course of the haemangioma may be due to local pressure effects on the adjoining spinal cord and or repeated episodes of bleeding. Since the haemorrhage in cavernous haemangioma is venous and at low pressure, the presentation is not as dramatic as with arterial bleed.[7] Incidental asymptomatic lesions have also been reported. The neurological deterioration may have sometimes variable degree of recovery although most patients exhibit a gradual clinical decline. On histo-pathological correlation, four major clinical patterns have been defined : (a) acute episodes of stepwise deterioration with small but repeated haemorrhages or with thrombosis of the malformed vessels, (b) slow progression due to progressive enlargement of the cavernoma eventually with thicknening of the sinusoid vessels and gradual thrombosis, (c) acute onset with rapid deterioration due to intraparenchymal haemorrhages, (d) acute onset with gradual decline attributable to altered microcirculation due to intraparenchymal haemorrhage.[12] The present case had spontaneous recovery on three occasions before experiencing a gradual deterioration of neurological status, which could be attributed to small bleeds and progressive enlargement of the cavernoma. Cavernous angiomas are angiographically occult.[13],[14] MRI is diagnostic. The typical MR characteristics of cavernomas are reticulated mixed signal areas on both T1WI and T2WI, surrounded by low signal intensity predominant in T2WI. The enhancement is modest and cord swelling is occasional.[15] The hyperintense signal may represent old clot or haemosiderin. The present case had a well defined intramedullary lesion of predominantly low intensity on MRI, admixed with smaller areas of high intensity signal, and expansion of the cord.

The total surgical resection under high magnification using microsurgical techniques is a procedure of choice for the management of symptomatic intramedullary cavernous angioma.[16] Dissection must be performed by making myelotomy directly over the bluish discolouration of lesion and limited to surrounding haemosiderin stained gliotic plane to avoid injury to normal surrounding neural tissue. Subtotal removal may lead to reappearance of symptoms and continued progressive deterioration, as a result of bleeding from residual malformation.[11],[12] Radio surgery has no role in the treatment of cavernous angioma.

References